Dilated cardiomyopathy is characterized by an enlarged and poorly contracting left ventricle. The main causes include inflammatory, toxic, metabolic, inherited, idiopathic and miscellaneous factors. On evaluation, patients typically present with decreased cardiac output, tachycardia and signs of congestion. Tests include echocardiogram, which shows increased left ventricular size and reduced ejection fraction, and ECG, which may show conduction delays. Treatment focuses on managing symptoms with diuretics and blocking neurohormonal activation to prevent worsening, while devices like ICDs are used in eligible patients.

1. dilated
CARDIOMYOPATHIES

2. Definition
 Cardiomyopathies are defined as
"a heterogeneous group of diseases of the
myocardium associated with mechanical
and/or electrical dysfunction that usually (but
not invariably) exhibit inappropriate ventricular
hypertrophy or dilatation and are due to a
variety of causes that frequently are genetic."

3. WHO Classification
anatomy & physiology of the LV
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Diastolic dysfunction
3. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
3. Unclassified
• Fibroelastosis
• LV noncompaction

4. Dilated Cardiomyopathy
•An enlarged left ventricle with decreased systolic function
as measured by left ventricular ejection fraction
characterizes dilated cardiomyopathy .
• Systolic failure is more marked
• Diastolic dysfunction, in the setting of marked volume
overload

5. MajorCauses of Dilated
Cardiomyopathy
 Inflammatory Myocarditis –
Infective/Noninfective
 Toxic
 Metabolic
 Inherited Metabolic Pathway Defects
 Familial
 Idiopathic
 Miscellaneous

6. Pathophysiology
 Brief primary injury such as infection or toxin exposure.
 Some myocytes may die during the initial injury, while
others survive only to have later programmed cell death,
(apoptosis).
 As the surviving myocytes hypertrophy to accommodate
the increased burden of wall stress,
 Local and circulating factors stimulate deleterious
responses that contribute to progression of disease,
even in the absence of further primary injury,Dynamic
remodeling and the amount of ventricular dilation.
 Mitral regurgitation commonly develops as the valvular
apparatus is distorted by ventricular dilation

8. DCM: Inflammatory
Infective
Reported with almost all types of infectious agents
 Viral- Co xsackie , adenovirus, HIV, hepatitis C
 Parasitic - T. cruzi— Chag as' dise ase , toxoplasmosis
 Bacterial- diphtheria, Spirochetal ,Bo re llia
 Rickettsial-Q fever
 Fungal -with systemic infection

9. DCM: Inflammatory
Noninfective
 Granulomatous inflammatory disease -Sarcoidosis
,Giant cell myocarditis
 Hypersensitivity myocarditis
 Polymyositis,
 Dermatomyositis
 Collagen vascular disease
 Peripartum cardiomyopathy
 Transplant rejection

10. DCM: Inflammatory
 Mechanism
 Direct tissue injury resulting from viral infection
 Immune mediated injury
 Viral infection in susceptible hosts may be a
proximate cause of cardiomyopathy

11. DCM: Peripartum
Diagnostic Criteria
 1 mo pre, 6 mos post
 Echo: LV dysfunction
 LVEF < 45%
 LVEDD > 2.7 cm/m2
Epidemiology/Etiology
 1:4000 women
Riskfactors - increased maternal age, increased
parity, twin pregnancy, malnutrition, use of
tocolytic therapy for premature labor, and
preeclampsia or toxemia of pregnancyJAMA

12. DCM: Peripartum
Proposed mechanisms:
 Inflammation - reflect increased susceptibility
to viral myocarditis or
 Autoimmune -myocarditis due to cross-
reactivity of anti-uterine antibodies against
cardiac muscle.
 prolactin cleavage fragment, salt ingestion
 Women with full recovery are more likely to
tolerate a subsequent pregnancy than are

13. DCM: Toxic
 Alcohol
 Catecholamines: amphetamines, cocaine
 Chemotherapeutic agents: (anthracyclines, trastuzumab)
 Interferon
 Other therapeutic agents (hydroxychloroquine, chloroquine)
 Drugs of misuse (emetine, anabolic steroids)
 Heavy metals: lead, mercury
 Occupational exposure: hydrocarbons, arsenicals

14. DCM: Toxic
Alcoholic cardiomyopathy
 Toxicity is attributed both to alcohol and acetaldehyde.
 Superimposed vitamin deficiencies and toxic a additives
are rarely implicated.
 6 drinks daily for 5–10 years, but frequent binge
drinking may also be sufficient.
 Reversible with abstinence
 Mechanism?:
 Myocyte cell death and fibrosis
 Directly inhibits: mitochondrial oxidative
phosphorylation Fatty acid oxidation

15. DCM: Toxic
Anthracyclines
 Cause vacuolar degeneration and myofibrillar loss.
 Generation of reactive oxygen species involving heme
compounds is currently the favored explanation for myocyte
injury and fibrosis.
 Disruption of the large titin protein may contribute to loss of
sarcomere organization.
 Three different presentations
 Acute heart failure/ Early onset /The chronic presentation -
 Leads to a relatively nondilated ventricle, perhaps due to
fibrosis.
 Thus, the stroke volume may be severely reduced with an
ejection fraction of 30–40%,
 Therapy is suppression of "inappropriate" sinus tachycardia,
and attention to postural hypotension .

16. DCM: Metabolic
 Nutritional deficiencies: thiamine, selenium,
carnitine
 Electrolyte deficiencies: calcium, phosphate,
magnesium
 Endocrinopathy:
 Thyroid disease
 Pheochromocytoma
 Diabetes
 Obesity
 Hemochromatosis

17. DCM :Familial
 Skeletal and cardiac myopathy
 Dystrophin-related dystrophy (Duchenne's,
Becker's)
 Mitochondrial myopathies (e.g., Kearns-Sayre
syndrome)
 Arrhythmogenic ventricular dysplasia
 Hemochromatosis
 Associated with other systemic diseases

18. DCM :Familial
 30% of ‘idiopathic’
 Inheritance patterns
 Autosommal dom/rec, x-linked, mitochondrial
 Associated phenotypes:
 Skeletal muscle abn, neurologic, auditory
 Mechanism:
 Abnormalities in:
 Energy production
 Contractile force generation
 Specific genes coding for:
 Myosin, actin, dystophin…

19. Inherited Defects in Metabolic Pathways Associated
With Cardiomyopathy
Glycogen Storage Diseases
 II—Pompe's (alpha 1,4
glucosidase)
 III—Forbes: de-branching
enzyme (amylo 1,6 glucosidase)
Glucose Metabolism(Defective
PRKAG2a
 Fatty acid metabolism
 Carnitine transport defect
 Medium chain Acyl-CoA
dehydrogenase
 Long chain Acyl-CoA
dehydrogenase
 Sphingolipidoses
 Fabry's dise ase (alpha
galactosidase A)
 Gaucher disease (beta-
glucocerebroside)
 Disorders of lysosomal function
 Danon's disease—(lysosome-
associated membrane protein,
LAMP2)
 Miscellaneous
 Hemochromatosis—Fe metabolism
 Familial amyloidosis—abnormal
transthyretin
 Barth syndrome—tafazzin defect

20. Overlap with Restrictive
Cardiomyopathy
 "Minimally dilated cardiomyopathy"
 Hemochromatosis
 Amyloidosis
 Hypertrophic cardiomyopathy

21. DCM: Idiopathic
 Is a diagnosis of exclusion,
 when all other known factors have been
excluded.
 two-thirds of dcm are still labeled as
idiopathic;
 may reflect unrecognized genetic disease.
 Continued reconsideration of etiology often
reveals later

22. Miscellaneous
Arrhythmogenic RV Dysplasia
 Desmosomal complex disrupt myocyte junctions
and adhesions,
 Myocardium of RV free wall replaced:
 Fibrofatty tissue
 Regional wall motion/function is reduced
 Ventricular arrhythmias
 SCD in young
 "woolly hair," and thickened palms and soles.

23. Arrhythmogenic RV Dysplasia

24. Miscellaneous
LV Noncompaction
Diagnostic Criteria
 Prominent trabeculations, deep recesses in LV apex
 Thin compact epicardium, thickened endocardium
 associated with multiple genetic variants in the sarcomeric
and other proteins such as tafazzin
Prognosis and Treatment
 Increased risk of CHF, VT/SCD, thrombosis
 Hereditary risk
 Screening of offspring

25. Echo: LV Noncompaction

26. Miscellaneous
Tako-Tsubo Cardiomyopathy
 The apical ballooning syndrome, or stress-induced
cardiomyopathy, occurs typically
 In older women after sudden intense emotional or
physical stress
 Presentations include pulmonary edema, hypotension,
and chest pain with ECG changes mimicking an acute
infarction.
 May result from intense sympathetic activation with
heterogeneity of myocardial autonomic innervation,
diffuse microvascular spasm, and/or direct
catecholamine toxicity.

27. Tako-Tsubo Cardiomyopathy

28. Evaluation of the DCM
HISTORY
 Detailed family history
 History of alcohol, illicit drugs, chemotherapy or radiation
therapy
 A past or associated history of rheumatologic, endocrine, or
infectious diseases
 Assessment of ability to perform routine and desired
activities
 Assessment of volume status, orthostatic blood pressure,
body mass index

29. Physical Exam
Decreased C.O.
Tachycardia
↓ BP and pulse pressure
cool extremities (vasoconstriction)
Pulsus Alternans (end-stage)
Pulmonary venous congestion:
rales
pleural effusions
Cardiac:
laterally displaced PMI
S3
mitral regurgitation murmur
Systemic congestion
↑ JVD
hepatosplenomegaly
ascites
peripheral edema

30. Chemistry
 Serum sodium,
potassium,calcium,magnesiu
m
 Fasting glucose
 Creatinine,blood urea
nitrogen
 Albumin, total protein,
 Liver function tests
 Lipid profile
 Thyroid-stimulating hormone
 Serum iron, transferrin
saturation
Hematology:
 Hemoglobin/hematocrit
 White blood cell count
with differential,including
eosinophils
 Erythrocyte
sedimentation rate

31. SEROLOGY
 Acute viral (coxsackie virus, echovirus,
influenza virus)
 Human immunodeficiency virus,
 Chagas' disease,
 Lyme disease,
 Toxoplasmosis

32. X-RAY CHEST
 Cardiomegaly
 Pulmonary vascular
congestion
 Kerley B lines
 Prominent
vasculature of the
upper lung fields.
 Pleural effusion
usually on the right
side, but it can be
bilateral

33. Electrocardiogram
 No specific electrocardiographic findings
 Sinus tachycardia is often present
 poor R wave progression, intraventricular conduction
delays, and LBBB.
 A wide QRS complex portends a worse prognosis
 left ventricular fibrosis may exhibit anterior Q waves
 nonspecific ST-segment and T wave abnormalities as
well as P wave alterations
 Persistent supraventricular or ventricular
tachyarrhythmias represent an important etiologic factor
for ventricular dysfunction

34. ECHOCARDIOGRAPHY.
 Cornerstone in the evaluation and
management
 LVEDD are usually greater than 60 mm
 Global hypokinesia
 Decreased EF and FS
 Associated ds

35. ECHOCARDIOGRAPHY

36.  CARDIAC MRI AND MULTIDETECTOR CT
 RADIONUCLIDE IMAGING
 INVASIVE EVALUATION INCLUDING
 ENDOMYOCARDIAL BIOPSY.

37. Management
PHARMACOLOGIC ANDDEVICE THERAPY
 Neurohormonal antagonists to prevent
disease progression
 Diuretics to maintain the volume balance are
the therapeutic cornerstone
 Prophylactic implantable cardiac defibrillators
and biventricular pacemakers is indicated in
appropriate patients

38. CRT: Cardiac Resynchronization
Therapy
1. Improved hemodynamics
 Increased CO
 Reduced LV filling
pressures
 Reduced sympathetic
activity
 Increased systolic function
w/o MVO2
2. Reverse LV
remodeling/architecture
 Decreased LVES/ED
volumes
 Increased LVEF
 Circ ’02, JACC ’02, JACC
’02, NEJM’02

39. SURGERY.
 Patients with structural heart ds conditions
corrected
 Left ventricular assist devices- provide
aggressive mechanical support to patients
with advanced decompensated heart failure
EMERGING SPECIFIC THERAPIES
 infections and immunomodulatory agents
 Stem cells for cardiac regeneration and gene

40. Presentation with Symptomatic Cardiomyopathy
Dilated Restrictive Hypertrophic
Ejection fraction
(normal 55%)
Usually <30% 25-50% >60%
LVDD (nor<55 mm) 60 mm >60 mm (may be
decreased)
Often decreased
Left ventricular wall
thickness
Decreased Normal or increased Markedly increased
Atrial size Increased Increased; may be
massive
Increased; related to
abnormal
Valvular
regurgitation
Related to annular
dilation; mitral earlier,
during
decompensation;
tricuspid late stages
Related to endocardial
involvement; frequent
mitral and tricuspid
regurgitation, rarely
severe
Related to valve-
septum interaction;
mitral regurgitation
Common first
symptoms
Exertional intolerance Exertional intolerance,
fluid retention early
Exertional intolerance;
may have chest pain

41. THANKS !